Tratamiento quirúrgico del carcinoma de Merkel facial avanzado. Presentación de un caso / Surgical treatment of advanced facial Merkel cell carcinoma. A case report

Introducción. El carcinoma de Merkel es un tumor maligno poco frecuente, que afecta principalmente a la población caucásica y cuya etiología guarda relación con el poliomavirus de las células de Merkel. Conlleva mal pronóstico, especialmente en estadios finales. Caso clínico. Se expone el caso de una paciente que presentaba un tumor primario facial de grandes dimensiones, con avanzado grado de extensión, afectación linfática cervical y metástasis parotídea derecha. Fue tratada mediante exéresis de la lesión primaria y cobertura con injerto de piel parcial, linfadenectomía cervical y parotidectomía ipsilateral. Resultados. Se logró mejoría importante en la calidad de vida de la paciente y sobrevida de al menos seis meses. Conclusión. Aunque no está claro el manejo óptimo del carcinoma de Merkel avanzado debido a su mal pronóstico, la cirugía favorece una mejoría en la calidad de vida del paciente y puede tener un papel clave en el manejo del carcinoma de Merkel en los estadios avanzados.

Introduction. Merkel carcinoma is a rare malignant tumor that mainly affects the Caucasian population and whose etiology is related to the Merkel cell polyomavirus. It has a poor prognosis, especially in the final stages. Clinical case. The case of a patient who presented a large primary facial tumor, with an advanced degree of extension, cervical lymphatic involvement and right parotid metastasis is described. She was treated surgically by excision of the primary lesion and coverage with partial skin graft, cervical lymphadenectomy, and ipsilateral parotidectomy. Results. A significant improvement was achieved in the patient's quality of life and survival of at least six months.Conclusion. Although the optimal management of advanced Merkel carcinoma is unclear due to its poor prognosis, surgery improves the patient's quality of life and it can play a key role in the management of Merkel carcinoma in advanced stages.

Merkel cell carcinoma: epidemiology, clinical features, diagnosis and treatment of a rare disease

Abstract Merkel cell carcinoma is a rare skin cancer with neuroendocrine differentiation. The risk factors include sun exposure, advanced age, immunosuppression (such as transplant recipients, patients with lymphoproliferative neoplasms, or patients with HIV), and Merkel cell polyomavirus infection. Clinically, Merkel cell carcinoma appears as a cutaneous or subcutaneous plaque or nodule, but this tumor diagnosis is rarely made clinically. Therefore, histopathology and immunohistochemistry are usually necessary. Primary tumors without evidence of metastases are treated with complete surgical excision and appropriate surgical margins. The presence of occult metastasis in a lymph node is frequent and a sentinel lymph node biopsy should be performed. Postoperative adjuvant radiotherapy increases local tumor control. Recently, agents that block the PD-1/PD-L1 pathway have shown objective and durable tumor regression in patients with advanced solid malignancies. The first anti-PD-L1 antibody used in patients with Merkel cell carcinoma was avelumab, but pembrolizumab and nivolumab have also shown efficacy. This article describes the current state of knowledge of the epidemiology, diagnosis, and staging of Merkel cell carcinoma, as well as new strategies for its systemic treatment.

Quantification of human polyomaviruses MCPyV and HPyV6 in malignant and non-malignant skin lesions

Abstract Background: Human Polyomaviruses such as MCPyV and HPyV6 are frequently found as part of healthy skin microbiota and have been associated with Merkel cell carcinoma (MCC), pruritic and dyskeratotic dermatoses, respectively. Their presence in other types of skin conditions varies greatly depending on lesion type and population. Objectives: To analyse comparatively the presence of MCPyV and HPyV6 in nonmelanoma skin cancers and healthy skin. Methods: The authors utilized qPCR techniques to quantify these pathogens in NMSC, premalignant diseases, and healthy skin of 87 patients. Results: MCPyV was detected in over 40% of samples, while HPyV6 was in 9.6%. MCPyV load was higher in squamous cell carcinomas (SCC) compared to basal cell carcinomas (BCC) (p = 0.016) and HPyV6 showed a higher percentage of infected cells in areas of low solar exposure as well as normal skin (p = 0.012). A fair agreement (kappa = 0.301) was found between MCPyV detection in lesions and their respective perilesional skin, indicating a random process of local dissemination of the virus. Study limitations: The lack of a larger sampling of different lesion types and protein expression analyses limits the correlation findings. Conclusions: This is the first report of HPyV6 detection in the healthy skin of a Brazilian population, but the role of both polyomaviruses in NMSC has yet to be demonstrated.

Mohs micrographic surgery in rare cutaneous tumors: a retrospective study at a Brazilian tertiary university hospital

Abstract Background Mohs micrographic surgery is an established technique in the treatment of cutaneous neoplasms. It offers higher cure rates and the main indications are non-melanoma malignant skin tumors. Few studies have been performed on the treatment of rare tumors through this technique. Objective To study rare skin tumors and rare variants of basal cell carcinoma and squamous cell carcinoma submitted to Mohs micrographic surgery in a tertiary service in relation to frequency, disease-free evolution, and applicability of this surgical procedure for this group of tumors. Methods This was a retrospective observational study including rare skin tumors and less common variants of basal cell carcinoma and squamous cell carcinoma treated using Mohs micrographic surgery, between October 2008 and April 2021. Results During the study period, 437 tumors were treated using Mohs micrographic surgery, and 22 (5%) rare skin tumors were selected. The tumors comprised three dermatofibrosarcomas protuberans, two atypical fibroxanthomas, two spiradenomas, two hypercellular fibrohistiocytomas, one primary cutaneous adenocarcinoma, one trichoblastoma, one porocarcinoma, one chondroid syringoma, one cutaneous angiosarcoma, one Merkel cell carcinoma, and one sebaceous carcinoma. Six other cases of rare basal cell carcinoma variants with trichoepitheliomatous differentiation, metatypical basal cell carcinoma, and clear cell squamous cell carcinoma were included. There were no cases of recurrence after an average of six years of follow-up. Study limitations This is a retrospective study on rare neoplasms carried out in a single referral center, and this surgical technique isn't widely available in the public service. Conclusion This retrospective case series showed that Mohs micrographic surgery is an appropriate treatment for rare skin tumors. They corresponded to 5% of the tumors treated by the technique during a 12-year-period, with no recurrences identified.

Treatment and Outcome of Merkel Cell Carcinoma in A Single Center / 肿瘤防治研究

Objective To investigate the clinical features, treatment, and outcome characteristics of patients with Merkel cell carcinoma. Methods The clinical manifestations, laboratory tests, diagnosis and treatment, and follow-up data of six patients with Merkel cell carcinoma were retrospectively analyzed. Results Among the six patients with Merkel cell carcinoma, four were males and two were females, with a median age of 66 years old (57-76 years old). All six patients presented with skin swelling, and the clinical stages were as follows: stageⅠ in three patients, stage Ⅲ in one patient, and stage IV in two patients. Two patients were treated with surgery alone, three patients with surgery combined with radiotherapy and/or chemotherapy, and one patient with immunotherapy combined with chemotherapy. Until the follow-up time, four patients had no disease progression, one patient died because of disease progression, and one patient remained under treatment. Conclusion Limited-stage Merkel cell carcinoma is primarily treated with surgery and radiotherapy, meanwhile, metastatic Merkel cell carcinoma needs systemic therapy, and first-line immune checkpoint inhibitors targeting PD-1/ PD-L1 pathway can achieve better therapeutic results.

Merkel cell polyomavirus and its etiological relationship with skin tumors

Abstract Viruses have been frequently identified in several human neoplasms, but the etiological role of these viruses in some tumors is still a matter of controversy. Polyomaviruses stand out among the main viruses with oncogenic capacity, specifically the Merkel cell polyomavirus (MCPyV). Recent revisions in the taxonomy of polyomaviruses have divided the Polyomaviridae family into six genera, including 117 species, with a total of 14 currently known human-infecting species. Although the oncogenicity of polyomaviruses has been widely reported in the literature since 1950, the first description of a polyomavirus as an etiological agent of a neoplasm in humans was made only in 2008 with the description of MCPyV, present in approximately 80% of cases of Merkel cell carcinoma (MCC), with the integration of its genome to that of the tumor cells and tumor-specific mutations, and it is considered the etiological agent of this neoplasm since then. MCPyV has also been detected in keratinocyte carcinomas, such as basal cell carcinoma and squamous cell carcinoma of the skin in individuals with and without immunosuppression. Data on the occurrence of oncogenic viruses potentially involved in oncogenesis, which cause persistence and tissue injury, related to the Merkel cell polyomavirus are still scarce, and the hypothesis that the Merkel cell polyomavirus may play a relevant role in the genesis of other cutaneous carcinomas in addition to MCC remains debatable. Therefore, the present study proposes to explore the current knowledge about the presence of MCPyV in keratinocyte carcinomas.

Carcinoma primario de células de Merkel vulvar

Las células de Merkel se describieron originalmente en el estrato basal de la epidermis, con propiedades neuroendocrinas. El carcinoma de células de Merkel de la vulva es una neoplasia extremadamente rara y altamente agresiva. Existen pocos casos de estos tumores, la mayoría de los cuales han sido considerados tumores neuroendocrinos. El origen histológico y la etiología de esta enfermedad son controvertidas. Debido a su rareza en esta localización, no está claro si se comporta de manera diferente a los carcinomas de piel similares en otras localizaciones. Se presenta un caso de carcinoma primario de células de Merkel vulvar. El examen de biopsia por escisión reveló una tumoración de 4 x 3 centímetros en el tercio posterior del labio mayor izquierdo de la vulva sin afectación de los ganglios linfáticos. La paciente fue sometida a vulvectomía radical y disección bilateral de ganglios linfáticos inguinales. La evaluación histológica postoperatoria no mostró metástasis regionales ni distantes.

Merkel cells were originally described in the stratum basale of the epidermis with neuroendocrine properties. Merkel cell carcinoma of the vulva is an extremely rare and highly aggressive neoplasm. There are few cases of these tumors, most of which have been considered neuroendocrine tumors. The histologic origin and etiology of this disease are controversial. It is known for his aggressive behavior and propensity for early diffusion. Because of its rarity in this location, it is unclear whether it behaves differently from similar skin carcinomas in other locations. A case of primary vulvar Merkel cell carcinoma is presented. Excisional biopsy examination revealed a 4 x 3-centimeter tumor in the posterior third of the left labium majus of the vulva without lymph node involvement. The patient underwent radical vulvectomy and bilateral inguinal lymph node dissection. Postoperative histological evaluation showed no regional or distant metastases.

Carcinoma de células de Merkel: múltiplas lesões cutâneas primárias no membro inferior / Merkel cell carcinoma: multiple primary skin lesions in the lower limb

Carcinoma de células de Merkel é um tumor neuroendócrino raro e agressivo de pele que usualmente apresenta-se como lesão única na região de cabeça ou pescoço. Relata-se um caso de topografia e apresentação atípicas, com presença de múltiplos e simultâneos tumores na perna esquerda de rápida evolução, associados à linfonodomegalia inguinal palpável, com diagnóstico confirmado por meio de histopatologia e imuno-histoquímica. Realizada exérese de linfonodo inguinal esquerdo e das lesões cutâneas com margem de segurança

Merkel cell carcinoma is a rare and aggressive neuroendocrine skin tumor usually presenting as a single lesion in the head or neck region. We report a case of atypical topography and presentation, with multiple and simultaneous tumors on the left leg of rapid progression associated with palpable inguinal lymphadenopathy and diagnostic confirmation by histopathology and immunohistochemistry. Exeresis of the left inguinal lymph node and skin lesions with a safety margin was performed

Progress of the pathogenesis in Merkel cell carcinoma / 肿瘤研究与临床

Merkel cell carcinoma (MCC) is a rare primary neuroendocrine carcinoma of the skin, which is named for its ultrastructure and immunophenotype similar to Merkel cells in the skin. It has been found that the integration of MCC with the oncogenic Merkel cell polyomavirus (MCPyV) may drive tumorigenesis or cause somatic mutations to the development of MCC because of ultraviolet ray-induced DNA damage. However, the pathogenesis of MCC is still unclear. This article introduces the current research progress of the pathogenesis of MCC, hoping to provide theoretical guidance for follow-up researches.

Carcinoma de Células de Merkel Metastático: Relato de Caso / Metastatic Merkel cell Carcinoma: Case Report / Carcinoma de Células de Merkel Metastásico: Relato de Caso

Introdução: O carcinoma de células de Merkel é um raro tumor neuroendócrino cutâneo, que se origina das células responsáveis pela sensibilidade tátil, possui caráter agressivo, evolução rápida e difícil tratamento. Relato do caso: Paciente do sexo masculino, 49 anos, caucasiano, que, ao atendimento dermatológico, apresentou nódulo indolor, infiltrando tecidos profundos, não ulcerado e localizado na região do braço esquerdo. O resultado da biópsia incisional foi positivo para carcinoma de células de Merkel. Após ressecção da lesão, os exames complementares evidenciaram doença metastática na axila e parede torácica. Com o tratamento quimioterápico, houve um benefício inicial com redução tumoral, porém, não durável, uma vez que foram reveladas novas áreas com metástases tumorais em regiões superiores do corpo, sendo submetido a novo procedimento cirúrgico, o qual, após novo regime quimioterápico, não obteve sucesso. Conclusão: Na ocasião do tratamento desse paciente, os anticorpos monoclonais, como o avelumab, não estavam disponíveis. O diagnóstico precoce com cirurgia de exérese da lesão imediata, antes do acometimento de outras regiões, permanece sendo a melhor opção para um prognóstico favorável ao paciente. Contudo, a despeito disso, com as limitações à época do tratamento, o paciente evoluiu a óbito.

Introduction: The Merkel cell carcinoma is a rare cutaneous neuroendocrine tumor that originates from cells responsible for tactile sensitivity, it has an aggressive character, fast evolution and difficult treatment. Case report: 49 years Caucasian male patient, with a painless nodule, infiltrating deep tissue, not ulcerated and located in left arm identified during the dermatological consultation. The result of the incisional biopsy was positive for Merkel cell carcinoma. After resection of the lesion, complementary exams revealed metastatic disease in the axilla and chest wall. The chemotherapy treatment brought an initial improvement with tumor reduction, however, it was not durable, because new areas with tumor metastases in upper regions of the body were revealed, the patient was submitted to an another surgical procedure, after which a new chemotherapy regimen failed. Conclusion:At the time of the treatment of this patient, monoclonal antibodies, such as avelumab, were not available. Early diagnosis with immediate lesion excision surgery, before the involvement of other regions, remains the best option for a better prognosis. However, regardless of this, because of the limitations at the time of the treatment, the patient died.

Introducción: El carcinoma de células de Merkel es un tumor neuroendocrino cutáneo raro, que se origina en células responsables de la sensibilidad táctil, tiene un carácter agresivo, una evolución rápida y un tratamiento difícil. Relato del caso: Paciente masculino, de 49 años, caucásico, que en atención dermatológica encontró nódulo indoloro, infiltrando tejidos profundos, no ulcerados y ubicados en la región del brazo izquierdo. El resultado de la biopsia incisional fue positivo para el carcinoma de células de Merkel. Después de la resección de la lesión, los exámenes complementarios mostraron enfermedad metastásica en la axila y la pared torácica. Con el tratamiento de quimioterapia, hubo un beneficio inicial con la reducción del tumor, sin embargo, no es duradero, ya que se revelaron nuevas áreas con metástasis tumorales en las regiones superiores del cuerpo, que se sometieron a un nuevo procedimiento quirúrgico, que después de un nuevo régimen de quimioterapia no tuvo éxito. Conclusión: En el momento del tratamiento de este paciente, los anticuerpos monoclonales, como avelumab, no estaban disponibles. El diagnóstico temprano con cirugía para la escisión de la lesión inmediata, antes de la participación de otras regiones, sigue siendo la mejor opción para un pronóstico favorable para el paciente. Sin embargo, a pesar de esto, con las limitaciones al momento del tratamiento, el paciente falleció.

Merkel cell carcinoma metastatic to the testis: report of a rare diagnosis and review of the literature

Merkel cell carcinoma is an aggressive malignancy that frequently recurs/disseminates, but metastases to the genitourinary tract are rare. Only eight cases of Merkel cell carcinoma metastatic to the testis are reported. We describe the ninth case of this event and provide a review of the literature. A 58-year-old man diagnosed with Merkel cell carcinoma of the wrist, presented, 37 months later, a recurrence in the form of a testicular metastasis. The tumor consisted of a monotonous proliferation of small, blue, round cells, with immunoexpression of neuroendocrine markers and the typical dot-like paranuclear immunostaining for cytokeratin 20, in the absence of immunostaining for cytokeratin 7. The patient is alive with no evidence of disease. Clinicians should be aware of the possibility of metastatic dissemination to the testis since genital examination/imaging is not part of routine follow-up for these patients, but timely orchiectomy may be curative.

Evidence and Considerations on Treatment of Small Size Merkel Cell Head and Neck Carcinoma

Abstract Introduction Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine malignant cancer. It is an epidermal cancer common in the head and neck. Objectives Though there is limited number of cases described in the literature for the treatment difficult to obtain. Our purpose was to present the clinical course and treatment of four patients with MCC. Methods We conducted a retrospective analysis and obtained detailed clinical information for all 4 patients treated for MCC at the ENT Department of the SS Annunziata Hospital in Chieti, Italy, from 2013 through 2015. Results In our study, two patients presented with the tumor in a rare site (lower eyelid). All of the patients underwent surgical treatment: three patients had free excision margins and negative sentinel lymph nodes (SLNs) while 1 patient had free excision margins and positive SLNs. The latter patient underwent ipsilateral neck dissection. In another patient, the fluorodeoxyglucose positron emission topography (FDG PET)/computed tomography (CT) performed 6 months after the surgery has shown high metabolic activity in the left parotid gland, and the patient underwent total parotidectomy and a neck dissection. Conclusion Sentinel lymph node biopsy is a useful technique in small size MCCs of the head and neck. However, the parotid gland should be strictly controlled in patients with lower eyelid tumors.

Merkel cell carcinoma: Preparing to go the distance

@#Merkel cell carcinoma (MCC) is a rare and aggressive malignancy of the skin, with poor clinical outcomes. Typical conditions include a rapidly growing, solitary dome-shaped, violaceous nodule. Several root causes have been identified - sun exposure, age, lighter skin, immunocompromised state, and polyomavirus infection. Wide local excision is the best treatment. The tumour is radiotherapy-responsive. However, the success rate of the treatment with chemotherapy is rather limited. Immunotherapy has shown promising results. Early detection is important to prevent morbidity and mortality. Case Report: In this literature work, we reported on a particular case of MCC, as exhibited by an 84-year-old Chinese woman, and discussed the clinical features and management of MCC. Discussion: We highlighted that MCC cases have a link to the polyomavirus 5. Patients who were identified with the Polyomavirus 5, and underwent immunotherapy, were seen to depict much better prognosis.

Carcinoma de células de Merkel de región glútea. Reporte de un caso / Merkel cell carcinoma of the gluteal region. Report of a case / Carcinoma de células de Merkel de região glútea. Relato de caso

Resumen: Introducción: el carcinoma de células de Merkel es un tumor primario maligno de piel que afecta fundamentalmente regiones expuestas a las radiaciones solares. Pocos casos han sido comunicados en una región no expuesta al factor de riesgo mencionado, como es la glútea. El objetivo de este trabajo es comunicar un caso clínico de carcinoma de células de Merkel de región glútea. Caso clínico: paciente de 63 años, sexo masculino, que consultó por tumoración de 2 cm de diámetro en región glútea derecha, de rápido crecimiento, que se operó de coordinación con anestesia, realizándose su resección completa. El resultado del estudio anatomopatológico informó carcinoma de células de Merkel. No se evidenció diseminación a distancia, por lo que de acuerdo con la clasificación TNM se determinó como estadio I. Se completó el tratamiento con radioterapia local. Actualmente el paciente sigue en seguimiento y asintomático. Discusión: los carcinomas de células de Merkel son tumores altamente agresivos. Además de la radiación solar como factor de riesgo, se mencionan la inmunodeficiencia y un nuevo poliomavirus, el poliomavirus de células de Merkel. Se presentan como tumoraciones de color violáceo y rápido crecimiento, y es frecuente el compromiso ganglionar sincrónico o metacrónico. La resección quirúrgica con márgenes suficientes y vaciamiento ganglionar, en caso de haber compromiso o estudio de ganglio centinela en caso de no haberlo, es lo indicado. El pronóstico depende del estadio y se ha reportado hasta 30% de recidiva a dos años.

Summary: Introduction: Merkel cell carcinoma is a malign primary tumour that mainly affects regions that are exposed to solar radiation. Few cases have been reported in a region that is not exposed to the above mentioned risk factor, as the gluteal regions. The study aims to communicate the clinical case of a Merkel cell carcinoma of the gluteal region. Clinical case: 63-year-old male patient who consulted for a tumour with a 2cm diameter in the right gluteal region, rapidly growing, being completely resected in a cordinaterd surgery under anesthesia. Pathology study revealed that it was Merkel cell carcinoma. No distance dissemination was seen, and thus as per the TNM staging system it was classified as stage I. Therapy was completed with local radiotherapy, It is currently under follow up and asymptomatic. Discussion: Merkel cell carcinoma are highly aggressive tumours. Apart from solar radiation as a risk factor, it is worth mentioning immunodeficiency and a new polyomavirus, the Merkel cell polyomavirus. This condition can be seen as purple, rapidly growing tumours and they frequently involve synchronic or metachronic lymph node compromise. Surgical resection with sufficient margins and lymph node emptying, in the event of lymph node compromise or sentinel node biopsy is recommended. Prognosis depends on the stage and a 30% relapse has been reported after 2 years.

Resumo: Introdução: o carcinoma de células de Merkel é um tumor primário maligno de pele que afeta fundamentalmente regiões expostas às radiações solares. São poucos os casos relatados em uma região não exposta a esse fator de risco, como é a glútea. O objetivo desta comunicação é apresentar o caso clínico de um carcinoma de células de Merkel de região glútea. Caso clínico: paciente de 63 anos, sexo masculino que consultou por tumoração de 2 cm de diâmetro, na região glútea direita, com rápido crescimento que foi ressecada completamente em uma cirurgia eletiva com anestesia. O laudo anatomopatológico foi: carcinoma de células de Merkel. Não se evidenciou disseminação a distância por isso foi classificado como estádio I de acordo com a classificação TNM. O tratamento foi completado com radioterapia local. Atualmente em seguimento e assintomático. Discussão: os carcinomas de células de Merkel são tumores altamente agressivos. Além da radiação solar, a imunodeficiência e um novo poliomavirus, o poliomavirus de células de Merkel, são mencionados como fator de risco. Apresentam-se como tumorações de cor violácea e crescimento rápido e frequentemente se observa compromisso ganglionar sincrônico ou metacrônico. A ressecção cirúrgica com margens suficientes e esvaziamento ganglionar, quando há compromisso ou estudo de gânglio sentinela quando não há, é a conduta indicada. O prognóstico depende do estádio e há registros de até 30% de recidiva a 2 anos.

Merkel cell carcinoma: A series of seven cases

BACKGROUND: Merkel cell carcinoma (MCC) is a rare neuroendocrine malignancy affecting the skin, for which timely diagnosis and aggressive treatment are essential. MCC has most often been reported in Caucasians, and case reports in Asians are rare. This study presents our experiences with the surgical treatment and radiotherapy of MCC in Asian patients. METHODS: We retrospectively reviewed the records of seven MCC patients between 2000 and 2018 from a single institution, and analyzed patient characteristics, tumor characteristics, surgical treatment, sentinel lymph node evaluation, reconstruction, adjuvant radiation therapy, and prognosis. RESULTS: Eight MCC lesions occurred in seven patients, most commonly in the head and neck region. All patients underwent surgical excision with reconstruction. The final surgical margin was 1.0 cm in most cases, and reconstruction was most commonly performed with a split-thickness skin graft. Five patients received adjuvant radiotherapy, and two patients received sentinel lymph node biopsy. During the follow-up period, three patients remained well, two died from other causes, one experienced recurrence, and one was lost to follow-up. CONCLUSIONS: We treated seven Asian MCC patients and our series confirmed that MCC is a very dangerous cancer in Asians as well. Based on our experiences, thorough surgical excision of MCC with histopathological clearance should be considered, with sentinel lymph node evaluation if necessary, followed by appropriate reconstruction and careful postoperative observation. Adjuvant radiation therapy is also recommended for all Asian MCC patients. The results of this case series may provide guidance for the treatment of Asian MCC patients in the future.

Two separate nodules of Merkel cell carcinoma occurring concomitantly on one cheek

Merkel cell carcinoma (MCC) is a rare and highly aggressive neuroectodermal carcinoma arising from mechanoreceptor Merkel cells. Multiple MCCs are even rarer. We report a case of two independent MCCs simultaneously present in the cheek of a patient, which were effectively and esthetically treated using a cheek flap. Punch biopsy performed in a 60-year-old woman admitted with a chief complaint of two skin-colored hard nodules in her left cheek, accompanied by an itching sensation, was suggestive of MCC. Accordingly, we performed sentinel lymph node biopsy through the modified Blair incision under general anesthesia, in cooperation with the head and neck surgery department. The defect was covered with a cheek flap by slightly extending the existing incision following wide excision with a safety margin of 1 cm. This paper is significant in that it introduces an effective reconstruction technique that maintains function using a cheek flap for the management of this rare case. In addition, this paper is the first to classify multiple MCCs according to the time of onset. We believe that this paper presents an effective alternative reconstruction technique with sentinel node biopsy through the modified Blair incision.

Upper eyelid Merkel cell carcinoma treated with neoadjuvant chemotherapy and surgical excision

Merkel cell carcinoma is a rare cutaneous carcinoma, featured by an aggressive clinical course and a mortality rate of 28% at 2 years. A 71-year-old female was affected by a 4.1-cm-wide locally advanced Merkel cell carcinoma of the upper eyelid, previously misdiagnosed as chalazion, with involvement of the extraocular muscles. Although the tumor showed a macroscopic spontaneous regression in size after the incisional biopsy, the mass was treated with neoadjuvant chemotherapy and surgical excision. Good functional and aesthetic result with preservation of the eyeball and absence of tumor recurrence were achieved at 3-year follow-up. In our experience, the combination of the inflammatory cascade due to the incisional biopsy and neoadjuvant chemotherapy led to the regression of a locally advanced large Merkel cell carcinoma of the eyelid.

Virus polioma en carcinoma de células de Merkel / Polyoma virus in Merkel cell carcinoma

Introducción: El carcinoma de células de Merkel (MCC) es un tumor cutáneo maligno agresivo y de mal pronóstico. La incidencia es mayor en adultos hombres, caucásicos, con edad promedio de 70 años. Feng et al, lograron aislar un nuevo virus en muestras de este tumor, que denominaron virus polioma de células de Merkel (MCPyV). Se ha intentado establecer una relación causal entre el virus y MCC. El virus está integrado al genoma y produciría mutaciones específicas. En muestras de MCC, se ha detectado expresión de oncoproteinas virales (antígenos T) que promueven la replicación viral y tumorogénesis

Introduction: Merkel cell carcinoma (MCC) is an aggressive malignant cutaneous tumor with poor prognosis. Most cases affect elder patient with an average of 70 years of age. Feng et al isolated a new virus, the Merkel cell carcinoma polyoma virus (MCPyV). A causal relationship between MCPyV y MCC has been established. The virus is integrated in the genome and pro-duces specific mutations. MCC samples show ex-pression of viral oncoproteins (T antigens) that promote viral replication and tumorogenesis.

Merkel Cell Carcinoma of Cheek: 2 Case Reports / 대한이비인후과학회지

Merkel cell carcinoma (MCC) is a rare aggressive skin cancer with the mortality rate of 33%. MCC was first described by Toker in 1972 as a trabecular cell carcinoma and is classified as a neuroendocrine tumor similar to small cell lung carcinoma and melanoma. The pathogenesis of MCC remains largely unknown. However, ultraviolet radiation and immunosuppression are likely to play a significant pathogenic role. The primary skin lesion is usually asymptomatic and it typically presents as a red or purple dome-shaped nodule. The management of MCC is controversial, however, local wide excision followed by radiotherapy is accepted as the primary treatment modality. The regional draining nodal basin is the most common site for recurrence. Therefore, sentinel lymph node biopsy is recommended in all cases, except for the clinically node-negative cases. We herein report two cases of MCC on the left cheek with different clinical manifestations.

Merkel Cell Carcinoma Originating in a Setting of Pre-existing Bowen's Disease / 대한피부과학회지

No abstract available.